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Clinical Impact of Spontaneous Hyperactivity in Degenerating Retinas: Significance for Diagnosis, Symptoms, and Treatment
Hereditary retinal degenerations result from varied pathophysiologic mechanisms, all ultimately characterized by photoreceptor dysfunction and death. Hence, much research on these diseases has concentrated on the outer retina. Over the past decade or so increasing attention has focused on concomitan...
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Frontiers Media S.A.
2018
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Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6139326/ https://www.ncbi.nlm.nih.gov/pubmed/30250425 http://dx.doi.org/10.3389/fncel.2018.00298 |
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author | Stasheff, Steven F. |
author_facet | Stasheff, Steven F. |
author_sort | Stasheff, Steven F. |
collection | PubMed |
description | Hereditary retinal degenerations result from varied pathophysiologic mechanisms, all ultimately characterized by photoreceptor dysfunction and death. Hence, much research on these diseases has concentrated on the outer retina. Over the past decade or so increasing attention has focused on concomitant changes in complex inner retinal neural circuits that process visual signals for transmission to the brain. One striking abnormality develops before the ultimately profound anatomic disruption of the inner retina. Highly elevated spontaneous activity was first demonstrated in central nervous system visual centers in vivo by Dräger and Hubel (1978), and subsequently has been confirmed in vitro, now in multiple animal models and by multiple investigators (see other contributions to this Research Topic). What evidence exists that this phenomenon occurs in human patients with retinal degeneration, and what is the ultimate effect of spontaneous hyperactivity in the output neurons, the retinal ganglion cells? Here I summarize abnormalities of visual perception among patients with retinal degeneration that may arise from hyperactivity. Next, I consider the disruption of neural encoding and anatomic connectivity that may result within the retina and in downstream visual centers of the brain. I then consider how specific characteristics of hyperactivity may distinguish various forms or stages of retinal degeneration, potentially helping in the near future to refine diagnosis and/or treatment choices for different patients. Finally, I review how consideration of these features may help optimize pharmacologic, gene, stem cell, prosthetic or other therapies to forestall visual loss or restore sight. |
format | Online Article Text |
id | pubmed-6139326 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2018 |
publisher | Frontiers Media S.A. |
record_format | MEDLINE/PubMed |
spelling | pubmed-61393262018-09-24 Clinical Impact of Spontaneous Hyperactivity in Degenerating Retinas: Significance for Diagnosis, Symptoms, and Treatment Stasheff, Steven F. Front Cell Neurosci Neuroscience Hereditary retinal degenerations result from varied pathophysiologic mechanisms, all ultimately characterized by photoreceptor dysfunction and death. Hence, much research on these diseases has concentrated on the outer retina. Over the past decade or so increasing attention has focused on concomitant changes in complex inner retinal neural circuits that process visual signals for transmission to the brain. One striking abnormality develops before the ultimately profound anatomic disruption of the inner retina. Highly elevated spontaneous activity was first demonstrated in central nervous system visual centers in vivo by Dräger and Hubel (1978), and subsequently has been confirmed in vitro, now in multiple animal models and by multiple investigators (see other contributions to this Research Topic). What evidence exists that this phenomenon occurs in human patients with retinal degeneration, and what is the ultimate effect of spontaneous hyperactivity in the output neurons, the retinal ganglion cells? Here I summarize abnormalities of visual perception among patients with retinal degeneration that may arise from hyperactivity. Next, I consider the disruption of neural encoding and anatomic connectivity that may result within the retina and in downstream visual centers of the brain. I then consider how specific characteristics of hyperactivity may distinguish various forms or stages of retinal degeneration, potentially helping in the near future to refine diagnosis and/or treatment choices for different patients. Finally, I review how consideration of these features may help optimize pharmacologic, gene, stem cell, prosthetic or other therapies to forestall visual loss or restore sight. Frontiers Media S.A. 2018-09-10 /pmc/articles/PMC6139326/ /pubmed/30250425 http://dx.doi.org/10.3389/fncel.2018.00298 Text en Copyright © 2018 Stasheff. http://creativecommons.org/licenses/by/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms. |
spellingShingle | Neuroscience Stasheff, Steven F. Clinical Impact of Spontaneous Hyperactivity in Degenerating Retinas: Significance for Diagnosis, Symptoms, and Treatment |
title | Clinical Impact of Spontaneous Hyperactivity in Degenerating Retinas: Significance for Diagnosis, Symptoms, and Treatment |
title_full | Clinical Impact of Spontaneous Hyperactivity in Degenerating Retinas: Significance for Diagnosis, Symptoms, and Treatment |
title_fullStr | Clinical Impact of Spontaneous Hyperactivity in Degenerating Retinas: Significance for Diagnosis, Symptoms, and Treatment |
title_full_unstemmed | Clinical Impact of Spontaneous Hyperactivity in Degenerating Retinas: Significance for Diagnosis, Symptoms, and Treatment |
title_short | Clinical Impact of Spontaneous Hyperactivity in Degenerating Retinas: Significance for Diagnosis, Symptoms, and Treatment |
title_sort | clinical impact of spontaneous hyperactivity in degenerating retinas: significance for diagnosis, symptoms, and treatment |
topic | Neuroscience |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6139326/ https://www.ncbi.nlm.nih.gov/pubmed/30250425 http://dx.doi.org/10.3389/fncel.2018.00298 |
work_keys_str_mv | AT stasheffstevenf clinicalimpactofspontaneoushyperactivityindegeneratingretinassignificancefordiagnosissymptomsandtreatment |